Japanese clinicians have investigated cold-related paralysis in bulbospinal muscular atrophy (BSM). They showed that :
- they were frequent (88%) in an observational study of 51 patients with SBMA and 18 without the disease,
- mexiletine does not significantly reduce their frequency, results of the MEXPRESS crossover trial which involved 20 participants on mexiletine or placebo, taken three times a day for 4 weeks,
- however, mexiletine improved tongue pressure and scores on the 10-second grip test or the release test.
